Given that Liston et al., employing mice in which one Aire allele was deleted, found that PGE affects in quantitative terms the magnitude of self-reactive T cells escaping negative selection (296), it has been hypothesized that conditions of partial AIRE deficiency may represent a risk for non-syndromic autoimmunity when acting in synergy with other susceptibility factors. Here, AIRE is linked to hyperinsulinemic hypoglycemia, familial, 4.